Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease

Abstract

Acute neurological changes in sickle cell disease (SCD) patients often raise the suspicion for stroke. Posterior reversible encephalopathy syndrome (PRES) can mimic stroke in its clinical presentation. We aimed to (i) review the PRES literature in SCD patients including clinical presentation, risk factors, pathophysiology, and management and (ii) elucidate the distinction between PRES and stroke in SCD. The exact pathophysiology of PRES in SCD remains elusive but is likely multifactorial and related to sickling, ischemia, and chronic anemia predisposing to vasogenic edema. PRES and stroke in SCD are distinguishable conditions. Our review may help elucidate a clinical approach to this distinction.

Rare Case of Posterior Reversible Leukoencephalopathy Syndrome Secondary to Acute Chest Syndrome

“…Therefore, to our knowledge, we believe that our case is only the third reported case in literature in an adult with PRES secondary to sickle cell crisis [6]. It should be noted that cases reported as stroke in the literature were unrecognized PRES, and therefore a literature search would overlook these findings [3]. Investigating the difference between PRES from a stroke can be difficult, yet the distinction is important as cerebral infarction suggests irreversible tissue injury.…”

“…One hypothesis is that severe hypertension causes impaired cerebrovascular autoregulation, vasodilatation, and vasogenic edema [3]. Literature reviews on PRES and hypertension widely discuss the “endothelial hypothesis” as the pathophysiological cause for a patient's high blood pressure [4].…”

“…However, PRES appears to be related to endothelial dysfunction that leads to cerebrovascular autoregulation impairment and vasogenic edema. Evidently, since endothelial dysfunction is thought to play a part in the cerebrovascular disease observed in Sickle Cell (SC) anemia, SC patients may have an increased risk of developing PRES [3]. …”

“…Although seizures are the most common presentation, such as in our case presentation, PRES can also present with headache, visual disturbances, altered mental status, vomiting, ataxia, aphasia, or hemiparesis [3]. PRES is characterized by reversible radiographic signs of posterior leukoencephalopathy in the presence of headache, altered level of consciousness, and seizures with or without visual disturbances [5].…”

Neurological Complications and MRI

“…Risk factors include recent trauma, transfusion in the past fortnight, corticosteroid or non-steroidal anti-inflammatory use and intermittent hypertension [76]. Posterior reversible encephalopathy syndrome (Figure 4, left) has also been reported in the context of hypertension and cyclosporine use for nephrotic syndrome [77], as well as after acute chest syndrome [78,79]. Acute bilateral borderzone ischaemia may also occur secondary to inadequate global CBF to supply the tissue's demand for oxygen (e.g.…”